Background/Purpose:  Anemia in systemic lupus erythematosus (SLE) might have various etiologies; however, autoimmune hemolytic anemia (AIHA) is the characteristic hematologic feature. AIHA might be primary or have other etiologies as well. We evaluated the demographic, clinical features and prognosis of SLE patients with AIHA. In addition, their features and prognosis were compared to AIHA patients who were admitted to the Hematology Department of our institution.

Methods:  Data about SLE patients with AIHA were obtained from patients’ medical records. The diagnosis of SLE was based on 1997 revised ACR criteria. SLE patients with and without AIHA were compared to each other. In addition, medical records of patients with AIHA being followed up at the Hematology Department were screened for their clinical features and outcome.

Results: We included 333 SLE patients (307 females, 26 males, mean age: 37.7±12.4 years). AIHA was diagnosed in 20 patients (6%). Male SLE patients had more frequent AIHA than females (15.4% vs. 4.6%, p=0.04). Coombs test positivity was present in 60 patients (18%). Coombs positivity tended to be more frequent in male SLE patients (30.8% vs. 16.9%, p=0.1). SLE patients with AIHA had more frequent active disease (SLEDAI>4) (75% vs. 42.2%, p=0.006), accompanying thrombocytopenia (45% vs. 14.4%, p=0.003), renal involvement (50% vs. 28.5%, p=0.05), anti-dsDNA positivity (65% vs. 47.6%, p=0.12) and hypocomplementemia (70% vs. 33%, p=0.002). Contrarily, photosensitivity (40% vs. 69.5%, p=0.01) and arthritis (40% vs. 78.7%, p=0.001) were significantly less frequent in SLE patients with AIHA. Fortytwo patients (11.5%) had Coombs positivity without AIHA. Coombs (+) SLE patients had less frequent photosensitivity (53.3% vs. 71.1%, p=0.01); however, more frequent pleural involvement (25% vs. 14.3%, p=0.04), leucopenia (55% vs. 37.3%, p=0.01), lymphopenia (75% vs. 59.8%, p=0.03), anti-dsDNA positivity (70% vs. 44%, p=0.001), anti-ribozomal P positivity (11.9% vs. 4.5%, p=0.05), renal involvement (51.7% vs. 24.7%, p=0.001) and hypocomplementemia (67.3% vs. 27.7%,p=0.001). Two patients presented with AIHA during pregnancy. Survival of SLE patients with and without AIHA were not found to be different. When clinical features of fortyone AIHA patients (27 female, 14 male, mean age: 61.1±18 vs 35.4±10.5) diagnosed at the Hematology Department were compared to SLE patients with AIHA, the former group was significantly older (p<0.001). Being female (85% vs. 65.9%, p=0.1) and relapses (20% vs. 9.8%, p=0.2) tended to be more frequent in the SLE group. SLE patients with AIHA and other AIHAs had similar survival.

Conclusion:  AIHA was diagnosed in 6% of our SLE patients. AIHA was more frequent in males and seemed to be associated with active disease, renal involvement, and anti-dsDNA positivity. SLE patients with AIHA were significantly younger than AIHA patients being follwed up at the Hematology Department.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/general-features-of-sle-patients-presenting-with-autoimmune-hemolytic-anemia/